We report a 68-year-old female who presented to the cardiology clinic with ventricular tachycardia and specific finger nail abnormalities including proximal capillary loops and proximal and periungual erythema. The patient had multiple underlying connective tissue disorders and pulmonary fibrosis. Finger nail changes are highly specific and are an indication for all healthcare providers that connective tissue diseases (CTDs) are likely underlying. We review the clinical signs and symptoms, review diagnostic criteria, and further testing to evaluate for CTDs.The following core competencies are addressed in this article: Patient care, Medical knowledge.

We report a 68-year-old female who presented to the cardiology clinic with ventricular tachycardia with specific finger nail abnormalities. The patient had multiple underlying connective tissue disorders and pulmonary fibrosis. There are multiple finger nail changes that are indicative of connective tissue disorders. We review specific finger nail changes and provide a review of diagnostic criteria including clinical signs and symptoms and further tests needed to rule out or confirm underlying connective tissue disease (CTD).

CTDs are common, often overlap, require lifelong care and are associated with numerous organ involvement.[1] CTD commonly affects many organs, including skin and fingernails. In a case–control study by Tunc et al., 190 patients with known CTD were enrolled and compared against two control groups. For those with SLE, a control group of young healthy patients was used, whereas patients with other CTD were compared against healthy elderly controls.[2] The study found fingernail changes are highly specific for CTDs (98–100%) but have poor sensitivity (13–31%).[2] The results are summarized in [Table 1]. The study concluded that finger nail changes do not correlate with disease activity with the following two exceptions: Splinter hemorrhage-SLE and increase in transverse nail curvature-systemic sclerosis (SSc). Two additional studies have attempted to determine the frequency of nail-fold changes in patients with CTD. Both studies corroborate with the study by Tunc in that nail changes have poor sensitivity for CTD.[3],[4] Fingernail changes are not exclusive to CTD, and an alternative diagnosis should be sought if fingernail changes are seen outside of those listed in [Table 1].[5]

Reynaud's phenomena can also be associated with various CTD, including SSc. Nailfold videocapillaroscopy (NVC) and analysis of red blood cell velocity offers a noninvasive indicator that is both sensitive, specific, and prognostic for underlying SSc. Initially, capillary morphology and analysis with microscopy appeared to offer little relation between clinical features.[6] A specific classification of NVC has been developed by Carpentier and Maricq further modified by Bergman et al. to classify a pattern consistent with SSc.[7],[8] Cutolo et al. further sub classified the NVC into early, active, and late stage prognostic indicators that may be predictive of organ involvement in SSc based on capillary arrangement, quantity and level of organization.[9],[10] Red blood cell velocity offers an additional diagnostic tool separate from NVC. In a study by Mugii et al. analyzing patients who were healthy versus known SSc, even patients with normal NSV with SSc were found to have a 51.7% reduction in red blood cell velocity.[11]

The pathogenesis of fingernail changes from CTD involves antibodies which alter the underlying structure and vessel supply of the proximal nail and nailbed. When detecting the previously mentioned nail changes in [Table 1], the physician should attempt to elicit other signs and symptoms of CTD and perform further evaluation detailed in [Table 2].[12],[13],[14],[15],[16],[17] If clinical signs and symptoms are detected, further laboratory testing can be useful as outlined in [Table 3].[17] If underlying Reynaud's phenomena is detected, sending the patient for NVC and red blood cell velocity analysis is warranted for early diagnosis of underlying SSc. These test also have the additional benefit of prognostic indications of SSc.

Table 2: An Algorithm of further diagnostic studies when fingernail changes are detected

CTD can be difficult to diagnose, and often times patients can present with devastating signs and symptoms. However, the finger nail changes listed in [Table 2] are an indicator for further investigation and workup for underlying CTD. If the healthcare provider suspects underlying CTD in the absence of finger nail changes, a workup is still indicated due to poor sensitivity of such changes.